What is sickle cell anemia?

Sickle cell anemia is a hereditary disease in which red blood cells are shaped like sickles rather than disks. Sickle cell anemia arises from a mutation in the hemoglobin gene that causes the blood cells to assume an abnormal shape. Sickle-shaped red blood cells have trouble delivering oxygen to the tissues of the body, and they increase the risk of blood clots.

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An estimated two million Americans have at least one copy of the sickle cell gene. Sickle cell anemia is most common in people of African or Mediterranean heritage and is also seen in people of South American, Central American, Caribbean, and Middle Eastern ancestry (Source: NHLBI).

Symptoms, which range from infrequent and mild to severe and long term, begin to develop in late infancy. Symptoms include attacks of pain, difficulty breathing, fatigue, paleness, jaundice, and even strokes. These attacks of symptoms, called crises, are often severe enough to require hospitalization and can lead to long-term complications.

Although there is currently no cure for sickle cell anemia, its symptoms can be managed. Common treatments include folic acid supplements, pain medication, and fluid supplements, as well as vaccines and antibiotics to control infections. Blood transfusions may be given for severe crises. Sickle cell anemia has many possible complications, including infections, damage to organs, and psychological complications, all of which may require medical intervention. However, with current medical treatments, people with sickle cell anemia can expect to live full lives.

Emergency attention is needed for any severe sickle cell anemia crises or complications, including stroke. Seek immediate medical care (call 911) for symptoms of a serious crisis or serious infection, including chest pain, confusion or loss of consciousness for even a brief moment, sudden muscle weakness or paralysis, difficulty breathing, high fever, or changes in speech or vision.

Seek prompt medical treatment for symptoms of infection, including fever, aches and fatigue.

SYMPTOMS

What are the symptoms of sickle cell anemia?

Symptoms of sickle cell anemia include acute symptoms related to crises, including chest, bone, and abdominal pain, rapid heart rate (tachycardia), fatigue, fever, excessive thirst, and difficulty breathing. Other, longer-term symptoms include paleness, ulcers on the legs, jaundice, delayed puberty, and blindness. In some cases, sickle cell anemia can lead to stroke.... Read more about sickle cell anemiasymptoms

CAUSES

What causes sickle cell anemia?

Sickle cell anemia is a genetic condition inherited from both parents. Mutations in the gene that controls the body’s production of hemoglobin cause an abnormal form of the protein, called hemoglobin S, which leads to malformed red blood cells. These sickle-shaped red blood cells are less effective than the normal disk-shaped cells at circulating oxygen throughout the body. Sickle cells are also more fragile than normal red blood cells, leading to an increased risk of blood clots and increased cellular breakdown.... Read more about sickle cell anemiacauses

TREATMENTS

How is sickle cell anemia treated?

Although there is currently no certain cure for sickle cell anemia, the symptoms of a sickle cell crisis can be treated. Some people with the disease have mild and infrequent symptoms, but other individuals may have severe or even life-threatening crises. Long-term care for sickle cell anemia focuses on decreasing the frequency of crises and preventing complications. In some cases, a bone marrow transplant or stem cell therapy can provide a permanent treatment for sickle cell anemia, but neither of these treatments is an option for many people.... Read more about sickle cell anemiatreatments

Medical Reviewer: All content has been reviewed by board-certified physicians under the direction of Rich Klasco, M.D., FACEP. Last Annual Review Date: May 2, 2011 Copyright: © Copyright 2011 Health Grades, Inc. All rights reserved. May not be reproduced or reprinted without permission from Health Grades, Inc. Use of this information is governed by the HealthGrades User Agreement.

This Article is Filed Under: Heart, Blood and Circulation